关键词: Arrhythmogenic right ventricular cardiomyopathy Arrhythmogenic right ventricular dysplasia Magnetic resonance imaging Right atrial function Strain

来  源:   DOI:10.1186/s13244-024-01630-x   PDF(Pubmed)

Abstract:
OBJECTIVE: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by progressive fibrofatty infiltration of atrial and ventricular myocardium resulting in adverse cardiac events. Atrial function has been increasingly recognized as prognostically important for cardiovascular disease. As the right atrial (RA) strain is a sensitive parameter to describe RA function, we aimed to analyze the prognostic value of the RA strain in ARVC.
METHODS: RA strain parameters were derived from cardiac magnetic resonance (CMR) images of 105 participants with definite ARVC. The endpoint was defined as a combination of sudden cardiac death, survival cardiac arrest, and appropriate implantable cardioverter-defibrillator intervention. Cox regression and Kaplan-Meier survival analyses were performed to evaluate the association between RA strain parameters and endpoint. Concordance index (C index), net reclassification index (NRI), and integrated discrimination improvement (IDI) were calculated to assess the incremental value of RA strain in predicting the endpoint.
RESULTS: After a median follow-up of 5 years, 36 (34.3%) reaching the endpoint displayed significantly reduced RA strain parameters. At Kaplan-Meier analysis, impaired RA reservoir (RARS) and booster strains (RABS) were associated with an increased risk of the endpoint. After adjusting for conventional risk factors, RARS (hazard ratio [HR], 0.956; p = 0.005) and RABS (HR, 0.906; p = 0.002) resulted as independent predictors for endpoint at Cox regression analyses. In addition, RARS and RABS improved prognostic value to clinical risk factors and CMR morphological and functional predictors (all p < 0.05).
CONCLUSIONS: RARS and RABS were independent predictors for adverse cardiac events, which could provide incremental prognostic value for conventional predictors in ARVC.
UNASSIGNED: We evaluated the prognostic value of right atrial strain in ARVC patients and suggested cardiologists consider RA strain as a predictive parameter when evaluating the long-term outcome of ARVC patients in order to formulate better clinical therapy.
CONCLUSIONS: • Patients with ARVC had significantly reduced RA strain and strain rates compared with healthy participants. • Participants with lower RA reservoir and booster stains were associated with a significantly higher risk of adverse cardiac events. • RA booster and reservoir strain provide incremental value to conventional parameters.
摘要:
目的:致心律失常性右心室心肌病(ARVC)是一种遗传性心肌病,其特征是心房和心室心肌的进行性纤维脂肪浸润导致不良心脏事件。心房功能越来越被认为是心血管疾病预后的重要因素。由于右心房(RA)应变是描述RA功能的敏感参数,我们旨在分析RA菌株在ARVC中的预后价值。
方法:RA应变参数来自105名具有明确ARVC的参与者的心脏磁共振(CMR)图像。终点定义为心脏猝死的组合,存活心脏骤停,和适当的植入式心脏复律除颤器干预。进行Cox回归和Kaplan-Meier生存分析以评估RA应变参数与终点之间的关联。协调指数(C指数),净重新分类指数(NRI),并计算综合判别改善(IDI),以评估RA应变在预测终点时的增量值.
结果:经过5年的中位随访,达到终点的36(34.3%)显示出RA应变参数显着降低。在Kaplan-Meier分析中,RA储层受损(RARS)和加强菌株(RABS)与终点风险增加相关.在对常规风险因素进行调整后,RARS(危险比[HR],0.956;p=0.005)和RABS(HR,0.906;p=0.002)在Cox回归分析中作为终点的独立预测因子。此外,RARS和RABS提高了临床危险因素和CMR形态和功能预测因子的预后价值(均p<0.05)。
结论:RARS和RABS是心脏不良事件的独立预测因子,这可以为ARVC的常规预测因子提供增量预测价值。
我们评估了右心房劳损在ARVC患者中的预后价值,并建议心脏病专家在评估ARVC患者的长期预后时将RA劳损视为预测参数,以便制定更好的临床治疗方案。
结论:•与健康参与者相比,ARVC患者的RA应变和应变率显著降低。•具有较低RA储层和加强剂染色的参与者与不良心脏事件的风险显著较高相关。•RA助推器和储层应变为常规参数提供增量值。
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